Autoimmune polyendocrine syndrome type 3
| Autoimmune polyendocrine syndrome type 3 | |
|---|---|
| Other names | Autoimmune polyendocrinopathy type 3, Autoimmune polyglandular syndrome type 3, APS type 3, APS3, PAS3.[1] |
| Specialty | Endocrinology, Rheumatology |
| Symptoms | Any symptoms associated with its constituent diseases |
| Complications | Autoimmune thyroiditis (Always), Celiac Disease, Type I Diabetes, Autoimmune hypophysitis, Systemic Lupus Erythematosus, Sjögren's Syndrome, Vitiligo (May or May not be Present) |
| Usual onset | Any Age |
| Duration | Lifelong |
| Types | APS 3A: Autoimmune thyroiditis with Immune Mediated diabetes mellitus;
APS 3B: Autoimmune thyroiditis with Pernicious anemia; APS 3C: Autoimmune thyroiditis with Vitiligo and/or Alopecia and/or Another Organ Specific Disease |
| Causes | Combination of Genetic and Environmental Factors |
| Risk factors | Family History of APS III or other Autoimmune Diseases |
| Diagnostic method | Serum antibody assays; Autoimmune Thyroiditis Required for Diagnosis |
| Differential diagnosis | Autoimmune Polyendocrine Syndrome Type II, APECED, IPEX |
| Prevention | N/A |
| Treatment | Depends on Constituent Diseases |
| Prognosis | Normal Lifespan |
| Frequency | ~ 2-3% of population |
Autoimmune polyendocrine syndrome, type 3 is a condition characterized by the coexistence of autoimmune thyroiditis and at least one other autoimmune disease (excluding Addison's Disease).[2] Based on other organ-specific autoimmune involvement, there are multiple subtypes that are classified: type 3a shows thyroid autoimmune disease in conjunction with type 1 diabetes, type 3b shows thyroid autoimmune disease in conjunction with pernicious anemia (PA), and type 3c shows thyroid autoimmune disease in conjunction with alopecia, vitiligo, or other organ-specific autoimmune disease.[3]
Related Conditions
[edit]The hallmark of autoimmune polyglandular syndromes (APS) is the existence of autoimmune reactions directed against multiple endocrine and non-endocrine organs. There have been described as four primary types:[4]
- APS-1 [Autoimmune-Polyendocrine-Candidiasis-Ectodermal-Dystrophy Syndrome (APECED)]: Chronic hypoparathyroidism, autoimmune Addison's disease, and chronic candidiasis (two conditions must exist for the term to be used).[4]
- APS-2 (Schmidt's disease): Autoimmune Addison's disease (constantly present), in conjunction with either type 1 diabetes mellitus (DM) or autoimmune thyroid disease.[4]
- APS-3: Type 1 diabetes, atrophic gastritis, pernicious anemia, vitiligo, alopecia, and myasthenia gravis, and autoimmune thyroid disease; Addison's disease and/or hypoparathyroidism are not included in this association.[4]
- APS-4: Combinations that weren't in the earlier groups.[4]
Signs and symptoms
[edit]Most patients with autoimmune polyendocrine syndrome type 3 have autoimmune thyroid diseases associated with only one other autoimmune disease; these associations are most frequently with either type 1 diabetes (20–30% of cases)[5] or chronic atrophic gastritis (39 percent of cases).[6] Other disorders associated with autoimmune polyendocrine syndrome type 3 are pernicious anemia, vitiligo, alopecia, and myasthenia gravis.[4]
Epidemiology
[edit]Autoimmune polyendocrine syndrome, type 3 has a frequency of 1.4 to 2 per 100,000 people, with no discernible ethnic group preference.[7]
References
[edit]- ^ "Monarch Initiative". Monarch Initiative. Retrieved January 27, 2024.
- ^ Betterle, Corrado; Garelli, Silvia; Coco, Graziella; Burra, Patrizia (February 11, 2014). "A rare combination of type 3 autoimmune polyendocrine syndrome (APS-3) or multiple autoimmune syndrome (MAS-3)". Autoimmunity Highlights. 5 (1). Springer Science and Business Media LLC: 27–31. doi:10.1007/s13317-013-0055-6. ISSN 2038-0305. PMC 4389012. PMID 26000153.
- ^ Apolinario, Michael; Brussels, Aaron; Cook, Curtiss B.; Yang, Shaun (2022). "Autoimmune polyglandular syndrome type 3: A case report of an unusual presentation and literature review". Clinical Case Reports. 10 (2): e05391. doi:10.1002/ccr3.5391. ISSN 2050-0904. PMC 8815091. PMID 35140971.
- ^ a b c d e f Quintos, Jb; Grover, Monica; Boney, Charlotte M; Salas, Max (2010). "Autoimmune polyglandular syndrome Type 3 and growth hormone deficiency". Pediatric Diabetes. 11 (6): 438–442. doi:10.1111/j.1399-5448.2009.00622.x. S2CID 8964242.
- ^ Warncke, Katharina; Fröhlich-Reiterer, Elke E.; Thon, Angelika; Hofer, Sabine E.; Wiemann, Dagobert; Holl, Reinhard W. (June 14, 2010). "Polyendocrinopathy in Children, Adolescents, and Young Adults With Type 1 Diabetes". Diabetes Care. 33 (9). American Diabetes Association: 2010–2012. doi:10.2337/dc10-0404. ISSN 0149-5992. PMC 2928352. PMID 20551013.
- ^ Lahner, Edith; Centanni, Marco; Agnello, Giacoma; Gargano, Lucilla; Vannella, Lucy; Iannoni, Carlo; Delle Fave, Gianfranco; Annibale, Bruno (2008). "Occurrence and Risk Factors for Autoimmune Thyroid Disease in Patients with Atrophic Body Gastritis". The American Journal of Medicine. 121 (2). Elsevier BV: 136–141. doi:10.1016/j.amjmed.2007.09.025. ISSN 0002-9343. PMID 18261502.
- ^ Frommer, Lara; Kahaly, George J (April 26, 2019). "Autoimmune Polyendocrinopathy". The Journal of Clinical Endocrinology & Metabolism. 104 (10). The Endocrine Society: 4769–4782. doi:10.1210/jc.2019-00602. ISSN 0021-972X. PMID 31127843.
Further reading
[edit]- Betterle, C.; Furmaniak, J.; Sabbadin, C.; Scaroni, C.; Presotto, F. (2023-01-07). "Type 3 autoimmune polyglandular syndrome (APS-3) or type 3 multiple autoimmune syndrome (MAS-3): an expanding galaxy". Journal of Endocrinological Investigation. 46 (4): 643–665. doi:10.1007/s40618-022-01994-1. ISSN 1720-8386. PMID 36609775. S2CID 255501278.
- Horie, Ichiro; Kawasaki, Eiji; Ando, Takao; Kuwahara, Hironaga; Abiru, Norio; Usa, Toshiro; Yamasaki, Hironori; Ejima, Eri; Kawakami, Atsushi (June 1, 2012). "Clinical and Genetic Characteristics of Autoimmune Polyglandular Syndrome Type 3 Variant in the Japanese Population". The Journal of Clinical Endocrinology & Metabolism. 97 (6). The Endocrine Society: E1043–E1050. doi:10.1210/jc.2011-3109. hdl:10069/29379. ISSN 0021-972X. PMID 22466347.